PANDAS Network is dedicated to improving the diagnosis and treatment of children with PANDAS (Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections) and PANS (Pediatric Acute-onset Neuropsychiatric Syndrome). Armed with an impressive network of doctors, researchers and scientists, PANDAS Network strives to collaborate with subject matter experts, build public awareness, provide family support, and gather data and resources to better inform parents and the medical community about PANDAS and PANS.

What is PANDAS?

PANDAS (Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections) occurs when strep triggers a misdirected immune response and results in inflammation on a child’s brain. In turn, the child quickly begins to exhibit life changing symptoms such as OCD, anxiety, tics, personality changes, decline in math and handwriting abilities, sensory sensitivities, restrictive eating, and more.

Swedo described PANDAS in the 1990s while studying a childhood condition called Sydenham Chorea. The rare disorder can occur with rheumatic fever, the heart condition that can develop when a Group A strep infection goes untreated. Patients with Sydenham Chorea have rapid, irregular involuntary movements of the arms, legs, trunk, and facial muscles, in addition to psychiatric symptoms.

These diseases are very similar. Neurologists believe it affects the basal ganglia of the brain. Both illnesses (PANDAS and Sydenham Chorea) may be renamed 'basal ganglia encephalitits' by a consortium in 2019-2020.

PANDAS Network estimates that PANDAS/PANS affects as many as 1 in 200 children.

PANDAS CRITERIA

The hallmark trait for PANDAS is sudden acute and debilitating onset of intense anxiety and mood lability accompanied by Obsessive Compulsive-like issues and/or Tics in association with a streptococcal-A (GABHS) infection that has occurred immediately prior to the symptoms. In some instances, the onset will be 4 to 6 months after a strep infection because the antibiotics did not fully eradicate the bacteria. Many pediatricians do not know the latent variability of strep – Rheumatologists and Streptococcal Experts do.

When strep cannot be linked to the onset of symptoms, the NIMH states one should look into the possibility of PANS (Pediatric Acute-onset Neuropsychiatric Syndromes).

The acute onset means a Y-BOCS (Yale Brown Obsessive-Compulsive Scale) score of >20 and or a Chronic Tic Disorder YGTSS (Yale Global Tic Severity Scale) often with multiple tics. Below is the symptom criteria for PANDAS. Additional symptoms may be present.

A clinical diagnosis of PANDAS is defined by the following criteria:

Presence of significant obsessions, compulsions, and/or tics
Abrupt onset of symptoms or a relapsing-remitting course of symptom severity
Pre-pubertal onset
Association with streptococcal infection
Association with other neuropsychiatric symptoms

What is PANS?

PANS (Pediatric Acute-onset Neuropsychiatric Syndrome) is when an infectious trigger, environmental factors, and other possible triggers create a misdirected immune response results in inflammation on a child’s brain. In turn, the child quickly begins to exhibit life changing symptoms such as OCD, severe restrictive eating, anxiety, tics, personality changes, decline in math and handwriting abilities, sensory sensitivities, and more.

PANS was introduced in 2012 by Dr. Susan Swedo in the paper From Research Subgroup to Clinical Syndrome: Modifying the PANDAS Criteria to Describe PANS (Pediatric Acute-onset Neuropsychiatric Syndrome).

The PANS Criteria

PANS is a clinical diagnosis. The following is the “working criteria” as listed Dr. Swedo's paper on PANS:

Abrupt, dramatic onset of obsessive-compulsive disorder or severely restricted food intake.
Concurrent presence of additional neuropsychiatric symptoms, with similarly severe and acute onset, from at least two of the following seven categories: Anxiety Emotional lability and/or depression, Irritability, aggression and/or severely oppositional behaviors, Behavioral (developmental) regression, Deterioration in school performance, Sensory or motor abnormalities, Somatic signs and symptoms, including sleep disturbances, enuresis or urinary frequency
Symptoms are not better explained by a known neurologic or medical disorder, such as Sydenham’s chorea, systemic lupus erythematosus, Tourette disorder or others.

SYMPTOMS LIST:

Symptom presentation and severity can vary from child to child. It can also vary in each exacerbation. Below is a list of possible symptoms a child may exhibit. Not all need to be present. Not all possible symptoms are listed.

OBSESSIVE COMPULSIVE DISORDER (OCD)

OCD can manifest in different ways in young children.

RESTRICTIVE EATING
This includes selective eating and food refusal.There can a variety of reasons why the child experiences this, including contamination fears, sensory sensitivities, trouble swallowing, fear of vomiting or weight gain, and more. If restrictive eating is resulting in severe weight loss, call your provider immediately.

TICS
Tics are repetitive movements or sounds that can be difficult for a child to control. Motor tics can include eye-blinking, head-jerking, shoulder shrugging, nose-twitching, and facial grimacing. Some motor tics are a series of movements, performed in the same order. Vocal tics can include grunting, humming, throat clearing, coughing, repeating words or phrases. Some children are able to suppress tics temporarily, but doing so can cause extreme discomfort. Relief comes through performing the tic.

ANXIETY

EMOTIONAL LABILITY
Emotional lability includes not being able to control one’s emotional response such as uncontrollable crying or laughing. This is a neurological symptom.

DEPRESSION

IRRITABILITY AND AGGRESSION

BEHAVIORAL REGRESSION
This includes baby talk.

DEVELOPMENTAL REGRESSION

DETERIORATION IN SCHOOL PERFORMANCE
This includes deterioration in math skills, inability to concentrate, difficulty retaining information, and school refusal. School performance can also be a result of another contributing symptom, such as OCD or severe separation anxiety.

CHANGES IN HANDWRITING
This includes margin drifts and legibility.

SENSORY SENSITIVITIES
This can include being sensitive to touch, sounds, and noise. Simple touches may feel like they are hurting. For example: being unable to tolerate the way socks feel or the texture or temperature of certain foods. Sensory processing problems can also cause difficulty in finding an item when it is among a vast selection of items. For example, a child may have a hard time finding a shirt in a full dresser or finding words in a word search.

SOMATIC SIGNS
This includes sleeping difficulties, enuresis, frequent urination, and bed wetting.

HYPERACTIVITY

CHOREIFORM MOVEMENTS
Here is an example of Choreiform movements. The child is attempting to hold his hands straight out and is trying not to move his fingers.

SEVERE SEPARATION ANXIETY
Separation anxiety in an older child will present differently. For example, a child may be unwilling to leave the house or their bedroom.

HALLUCINATIONS
This includes both visual and auditory hallucinations.

FIGHT OR FLIGHT RESPONSE

DILATED PUPILS

RHEUMATIC PAIN OF JOINTS
Is often described.

URINARY PROBLEMS
This includes daytime wetting accidents and/or frequent urination.

Statistics

1 in 200 Children May Have PANDAS/PANS

PANDAS/PANS Prevalence

A conservative estimate is 1 in 200 children in the

U.S. alone. However, the true lifetime prevalence of PANDAS/PANS is not known.*

The statistical relevance is equal to Pediatric Cancer., Pediatric Diabetes I and II., and ALS³. The healthy outcome of a child’s life can be seriously affected, but research and treatment for PANDAS/PANS is scarce.

How do we determine the estimated lifetime prevalence of PANDAS/PANS for children 18 and under?

•Approximately 500,000 children are diagnosed with OCD in U.S.⁴

•Approximately 138,000 children are diagnosed with Tourette Syndrome in the U.S.⁵

•1.5 million+ children were diagnosed with serious anxiety/phobia/OCD/bipolar in a given year (1994-2011)⁶

•“Dr. Swedo estimates that (PANDAS) kids may make up as much as 25 percent of children diagnosed with OCD and tic disorders, such as Tourette syndrome.”⁷

*The estimated prevalence is based on PANDAS Network research.

PANDAS/PANS Population Analysis

Based on 700 family self-reports

AGE OF ONSET

1 to 3 years 11%
4 to 9 years 69%
10 to 13 years 19%
14+ years 1%

PRIMARY SYMPTOMS

OCD 37%
TICS 14%
BOTH 49%

INFECTIONS REPORTED

Strep 81%
Other 19%

(Mycoplasma, Lyme, etc.)

FAMILY MEDICAL HISTORY IN FIRST OR SECOND GENERATION

Based on 100 family self-reports 70% of families reported BOTH

Autoimmune Illness
Strep Related Severity Illness

PANDAS/PANS POPULATION OVERVIEW

Young age at onset: 6.5+/- 2 years for Tics and 7.4 +/- 2 years for OCD⁸
The ratio for boy to girls is 2.6:1 ; below age 8 years, the ratio of boys to girls is 4.7:1⁸

Citations

¹KantarHealth, CancerMPact Patient metrics U.S., accessed June 28, 2012

²National Diabetes Information Clearinghouse/NDIC (information as of 2010); http://1.usa.gov/LfNKrk

³ALS Association (information as of 2011); http://bit.ly/nRR94U

⁴IOCDF.org; http://bit.ly/rimFAb

⁵CDC.gov; http://1.usa.gov/MoghKH

⁶Centers for Disease Control and Prevention. Mental health surveillance among children - United States 2005–2011. MMWR 2013;62(Suppl; May 16, 2013):1-35.

⁷Scientific American: From Throat to Mind: Strep Today, Anxiety Later? (2010); http://bit.ly/5ro0mv

⁸Swedo et al (1998) PANDAS: Clinical Description of the First 50 Cases; http://ajp.psychiatryonline.org/data/Journals/AJP/3685/264.pdf

Diagnosis

PANDAS and PANS is a clinical diagnosis based on the collection of signs, symptoms, medical history, and laboratory findings that cannot be explained by any other neurological or medical disorders. Currently, there is not a 100% definitive test for PANDAS or PANS.

If you suspect your child has PANDAS or PANS, the following labs, in addition to completing the symptoms scales can help aid your medical provider in making a proper diagnosis.

BASIC BLOOD WORK:

IgE Level
IgA, IgM,
IgG (subclass 1, 2, 3, 4)
CBC
ANA
Ferritin
B-12
Vitamin D

VIRAL/BACTERIAL TESTING:

Strep throat culture, 48 hour culture or perianal culture
Bacteria & Virus Blood Work:
Antistreptolysin O (ASO)
Anti dNase B
Streptozyme
Lyme Disease and co-infections
Mycoplasma Pneumoniae IgA & IgM
Pneumococcal Antibody Titers
Epstein Barr Virus Panel
Coxsackie A & B Titers
HHV-6 Titers

ADDITIONAL TESTING:

Cunningham Panel* – autoimmune autoantibody levels: Dopamine D1 receptor, Dopamine D2L receptor, Lysoganglioside GM1, Tubulin, & CaM Kinase II.

The Complexity of Strep

INTRACELLULAR

The bacteria can adhere to the epithileal cells of the throat or nasal passages. Important to PANDAS cases: because at ONSET often the strep is reported as persistent and resistant to eradication. One child required nasal surgery. Strep was found in the biopsy.

A peer-reviewed article on Intracellular Strep:
(2008) Thulin, et al, How Group A Streptococci Hide in Macrophages

SOME SEROTYPES CAN BECOME MUCOID

The big culprit for Rheumatic Fevers is M18. If it develops a “mucoid shield” – typical antibiotics like penicillin or Augmentin cannot pierce the cell wall. Doctors must experiment with stronger antibiotics to rid the body of these.

The study below is highly complex – but it is shown here to illustrate the complexity of this bacteria:(2000) Cunningham, Pathogensis of Group A Streptococcal Infections.

MOLECULAR MIMICRY

This is a natural process by where the human body is attempting to rid itself of the strep bacteria. It is the causative factor for Rheumatic Fever and other virulent strep illnesses, and, probably – initially with PANDAS. Below are two studies attempting to explain this phenomena in PANDAS.

Below are two studies attempting to explain this phenomena in PANDAS.
Swedo and Grant (2004), Annotation: PANDAS: a model of human autoimmune disease.
Snider and Swedo (2003), Post-streptococcal autoimmune disorders of the central nervous system.

Non-Strep Triggers

Strep throat is very common in children. Typically, the symptoms are fever, sore throat, and white spots on tonsils. Some children present with an upset stomach, headache, and more. Additionally for some people, they may not exhibit any symptoms. For PANDAS children, the behavior changes are their indicating symptoms of a possible infection.

According to Dr. Susan Swedo, PANDAS children should not be classified as “strep carriers” since their PANDAS symptoms are an immune reaction. A person who is a classic strep carrier creates absolutely no negative reaction at all to the bacteria.

STREP IN OTHER PLACES OF THE BODY

In addition to the throat, strep can occur in the sinuses, in the ears, in the gut, on the skin, in the vagina, and peri-anal strep. A throat swab will not give you positive result for strep that is occurring somewhere else in the body.

STREP SWAB RAPID TEST AND CULTURE

A throat swab is the easiest and least invasive way to test for strep throat. If the child tests positive, treatment can begin quickly and the correlation between strep and the onset of symptoms can begin to be investigated.

If an in-office strep swab renders a negative test result make sure your office also CULTURES a swab since there is a chance for a false negative rapid test. There are some offices that will only do this upon request.

If your child or a family member tests positive for strep, schedule a follow up strep test two weeks after finishing all antibiotics.

TESTING FAMILY MEMBERS

It is important to swab all family members to be sure no one is asymptomatic when infected or a possible strep carrier. Carriers will often not show any strep symptoms, but if tested, will be positive for strep. A carrier will need one or two doses of antibiotics to rid themselves of strep.

Testing family members will lower your child’s chances of being re-infected. Also, some PANDAS children even react to exposure to strep.

STREP TESTING VIA BLOOD WORK

Strep ASO
Anti-DNase B Titer
Streptozyme

NORMAL TITERS DOES NOT ALWAYS MEAN STREP IS NOT PRESENT

In all literature regarding Rheumatic Fever, it is commonly known that strep has the ability to create “molecular mimicry” of the child’s own autoimmune response, therefore stopping the titer-rising process.

Also, according to the Shet et. al (2003) study:

Only 54% of children with strep showed a significant increase in ASO.
Only 45% showed an increase in anti–DNase B.
Only 63% showed an increase in either ASO and/or anti–DNase B.

In short, this means, not all children who have strep will have a rise in titers.

THE MAJORITY OF STREP INFECTIONS ARE MISSED

According to the Hysmith et. al (2017) study:

65% of new strep of new group A strep infections caused no symptoms yet were immunologically significant

Even though Strep is the most cited trigger for PANDAS and PANS, other bacteria, viruses, and environmental factors can create the misdirected immune response.

EXAMPLES OF NON-STREP INFECTIOUS TRIGGERS

MYCOPLASMA PNEUMONIAE

Mycoplasma Pneumoniae is also known as Walking Pneumonia

STAPH INFECTIONS

Staph can occur in multiple places in the body; on the skin, in the nose, and it has even been found in the biopsy results on tonsils post tonsillectomy

LYME DISEASE

The Traditional Western Blot should be done when Lyme is suspected. The Igenex Lyme test shows antibodies that MAY be present. Approach Igenex testing with a doctor who looks at this from a comprehensive understanding of the immune system and PANDAS/PANS.

INFLUENZA

COXSACKIE VIRUS

EPSTEIN BARR VIRUS

HERPES SIMPLEX VIRUS

OTHER POSSIBLE TRIGGERS

Once the autoimmune process is in place, other things may worsen or trigger symptoms in PANDAS/PANS children. Not every child will react to non-strep triggers. If your child is experiencing a flare of symptoms and no infection is present, the following are some possible suspects:

EXPOSURE TO ILLNESS

In PANDAS and PANS children, sometimes exposure to an illness can trigger symptoms.

ALLERGIES

This includes seasonal allergies.

STRESS

CHLORINE

DIET

Some families find changing the child’s diet helps. This may include ‘clean eating’, and eliminating certain foods such as gluten, dairy, etc.

YEAST OVERGROWTH

Antibiotics run the chance of also killing off the “good bacteria” in their body. This could result in an overgrowth of yeast or candida, which can occur in multiple places in the body, including the gut. If you suspect yeast overgrowth, discuss this your provider.

Immunological Workup

Some children with PANDAS or PANS may have underlying immunodeficiencies. The following tests are used in making that diagnosis. A clinical diagnosis of IgG deficiency may allow for insurance coverage for IVIG.

RECOMMENDED TESTS

CD4 This test measures generally over-reactive immune response.
IgG Subclass 1, 2, 3, 4 for total immunoglobulin levels
IgA and IgM

For an explanation of possible deficiencies, please reference IgG Subclass Deficiency and Specific Antibody Deficiency .

MRI/EEG/PET

MRI

A Pediatric Neurologist request an MRI. See the following for information on MRI as it relates to these disorders:

EEG

A few children have been found to have irregular EEG’s. This could be PANDAS or some other frontal lobe seizure issue which can create separate psychological or physical issues.

An EEG is also something to look into if a child experiences sleep abnormalities. A sleep study that results in abnormal findings may expedite receiving treatment.

PET SCAN

The Possible Future Test for PANDAS

In “Basal ganglia inflammation in children with neuropsychiatric symptoms” by Drs. Kumar, Williams, Musik and Chugani shows a significant difference in brain inflammation patterns between PANDAS children and those with Tourette Syndrome (TS). This information was obtained through special PET scanning.

These findings are very important because it reinforces the stance that PANDAS is not just Tourettes or OCD, but it is different and requires different treatment protocols. It also signifies the difference in the etiology, or cause, of the two disorders.

Basal Ganglia Imaging Study by Dr. Harry Chugani

The rogue white blood cells that cause PANDAS affect the basal ganglia of the brain. The basal ganglia are several brain structures located in the center of the brain. They function as a communication route to other parts of the brain and are partly responsible for the movement of our body. Other parts of the basal ganglia are involved in memorization, cognitive and emotional processing, and again initiating movement. There is still much to be learned about how this part of our brain functions. It is theorized that PANDAS causes an inflammation of the basal ganglia. A 1999 study by Ranjit C. Chacko, M.D. showed a connection between OCD and the basal ganglia.

Now, Dr. Harry Chugani at Children’s Hospital of Michigan, is studying the potential use of PET scans as diagnostic tools for PANDAS Dr Chugani states that the basal ganglia are not hard to detect on an MRI scan. In fact, the basal ganglia are large structures. However, the MRI scan is not useful for detection of abnormal microscopic, neurological functioning in the basal ganglia because MRI scans look at anatomy, i.e., tissue density and damage, water displacement, inflammation of tissue (and not the neurological function of this part of the brain). PET scans, or Positron Emission Tomography scans, may be used instead of, or in addition to, magnetic resonance imaging (MRI) scans. This gives anatomic and functional information.

The PET scan works by using PK-11195, a radioactive material, to target the inflammatory cells of the brain. This chemical is attracted to inflammatory cells and gives information on how the basal ganglia are functioning. Some of the PET scans on PANDAS children are identifying inflammation in the basal ganglia. This may be useful as the long awaited confirmation of PANDAS that parents have sought.

Dr Chugani said, “By finding PET scan evidence of abnormality in the basal ganglia, we may now have a biomarker for PANDAS Indeed, following IVIG in some PANDAS subjects, the abnormality in basal ganglia has gone away on repeated PET scan. These studies are rather preliminary, on a limited number of subjects, and much more work needs to be done.”

Cunningham Panel

THE TEST

The Cunningham Panel™ of Tests determines the “likelihood of the patient’s condition being autoimmune in nature”, including possible PANDAS and PANS. The test measure 5 assays and is commercially available by Moleculera Labs.

Dr. Cunningham’s research, which led to this test, measured cross-reactive antibodies that are elevated in a PANDAS child’s brains. Many children got assistance with PANDAS treatment from Immunologists if the results of this blood test indicate PANDAS-like antibody levels.

PHYSICIANS:

To order The Cunningham Panel™ of Tests within the United States, visit www.moleculeralabs.com.

INTERNATIONAL TESTING

Patients and Physicians outside the United States can find more details here, https://www.moleculeralabs.com/international-ordering-cunningham-panel/.

THE RESEARCH BEHIND THE TEST

In the following studies by Dr. Cunningham, et al., it is shown that movement disorders in Sydenham Chorea patients – a movement problem caused only by strep – and PANDAS children are similarly elevated. In both illnesses the basal ganglia are activated by a negative autoimmune reaction to strep.

This is important to show doctors so they understand that your child is not simply suffering from a psychological issue and may be having movement or mood issues due to strep antibody cross-reactivity from auto-antibodies called anti-lysoganglioside and anti-tubulin.

Symptom Scales

PANDAS/PANS SYMPTOM SCALE

PANDAS and PANS are clinical diagnoses that are heavily reliant on symptom presentation.

A clinical diagnosis is a diagnosis not solely based on a diagnostic test such as a blood test. Rather, the diagnosis is based on the collection of signs, symptoms, medical history, and laboratory findings. Currently, there is not a 100% definitive test for PANDAS or PANS.

Showing the severity of symptoms, the duration, and the onset are important tools in determining a possible diagnosis. The scale below will help parents present their child’s symptomatology.

The following scale is based on the clinical experience of Susan Swedo, M.D., Miroslav Kovacevic, M.D., Beth Latimer, M.D., and James Leckman, M.D., with the help of Diana Pohlman, Keith Moore and many other parents.

PEDIATRIC ACUTE NEUROPSYCHIATRIC SYMPTOM SCALE

OCD/TIC SCALES

The following scales are what doctors use to measure the severity of OCD and TICs. Review these scales and show them to your doctor and communicate that your child went from normal levels (1-5) to abnormal (10 and above) overnight. OCD may present as severe anxiety. Do not assume your child does not fall into the realm of OCD because they do not show the “stereotypical symptoms” of OCD, such as hand obsessive washing and fear of germs.

OCD Scale and Tic Scale

Treatment

Why Early Treatment Matters

Exacerbation can relapse and remit. They tend to increase in duration and intensity with each episode. Untreated PANDAS/PANS can cause permanent debilitation and in some cases can become encephalitic in nature. Repeat strep infections can cause serious problems. It is important to eradicate strep completely. Subsequent episodes can be caused by environmental and infectious triggers different from the original infection. Treated early and in a timely fashion, PANDAS/PANS can remit entirely.

Antibiotics

The type of infection dictates the type of needed antibiotic. If no improvement occurs over time, it may indicate a different antibiotic is needed or a different, or additional, infection is present.

Penicillin

This is a great first choice, because it is well studied prophylactic antibiotic for illnesses like Rheumatic Fever and Sydenham’s Chorea.

Augmentin
(Amoxicillin/Clavulanate Blend)

Cephalosporins
(Cephalexin, Cefdinir)

These have been very good for arresting most strains of strep. See remarks on Cephalexin superiority over penicillin regarding post-strep illnesses at page (3) of by Dr. R. Hahn, et al (2008) Evaluation of Post-streptococcal Illness.

The Cephalosporin alternative is also discussed in the American Academy of Pediatrics Journal, p. 1609 – Prevention of Rheumatic Fever and Diagnosis and Treatment of Acute Streptococcal Pharyngitis.

Azithromycin

Azithromycin is a front-line, broad based antibiotic that protects against many forms of bacteria.

Prophylactic Antibiotics

It has been recommended by physicians that the PANDAS child remain on prophylactic antibiotics in accordance to the RF (Rheumatic Fever) guidelines established by the American Academy of Pediatrics Journal. The RF guideline by the AAP is for 5 years after last attack or until age 21 (whichever is longer). According to the World Health Organization, the duration of prophylaxis for ARF is 5 years after last attack until 18 years old (whichever is longer).

Children who have had Sydenham’s Chorea, Rheumatic Fever or PANDAS have a risk of developing a more severe reaction upon reinfection with strep. Prophylaxis prevents reinfection.

Long term prophylactic antibiotic use for PANDAS has been shown in the study Antibiotic Prophylaxis with Azithromycin or Penicillin for Childhood-Onset Neuropsychiatric Disorders to “…play a role in the management of children in the PANDAS subgroup, as well as provide support for the assertion that GAS plays an etiologic role in some children with tics and/or obsessive-compulsive Disorder”. In that same study, it also states that “There was a 61% overall reduction in neuropsychiatric symptom exacerbations during the year of antibiotic prophylaxis and a 94% reduction in GAS triggered neuropsychiatric symptom exacerbations.”

According to the NIMH page , prophylactic antibiotics “may be helpful to use antibiotics as prophylaxis (prevention) against strep infections. Prophylactic antibiotics have proven to be quite beneficial to patients with rheumatic fever and Sydenham chorea”.

How PANDAS/PANS Children May React to Antibiotics

A Child That Improves With Antibiotics

The parents and provider will see improvement in the child and an easing of symptoms. Some children rapidly improve with antibiotics, while others experience improvement over a period of time. Children may have mild setbacks with viruses, other infections, etc. as the healing process continues.

A Child That Does Not Improve With Antibiotics

When a child has no improvement with antibiotics, the child’s episode may be encephalitic in nature and the inflammatory reaction may not only increase over time, but take many months to stop. A significant number of the original PANDAS cases PANDAS Network followed fit into this category.

For some children, antibiotics are needed for a minimum of 6 weeks to see a large reduction. Even then, there may not be a 100% reduction in symptoms. The autoimmune process is well in place and if after several months the child seems to be reactive to many illnesses at school or at home, it may be time to consider other treatment alternatives.

IVIG

Overview

IVIG is an intravenous pooled blood product comprised of immunoglobulins, that is used in treating immune deficiencies, encephalitis, and other medical conditions. The Immune Deficiency Foundation has more information on IVIG here

Drs. Perlmutter and Swedo used IVIG in the 1999, Lancet, Therapeutic plasma exchange and intravenous immunoglobulin for obsessive-compulsive disorder and tic disorders in childhood, where all of the children benefited from its use.

IVIG is endorsed for treating PANDAS by a consortium of physicians and researchers, as it is an autoimmune irregularity that causes encephalitic-like inflammation.. The PANDAS Physicians Network gives recommended dosing.

Healing is gradual over several months. Any form of infection will exacerbate a child while healing so manage exposures and illnesses carefully. Guidelines have been published in the Journal of Child and Adolescent Psychopharmacology (Feb 2015).

The majority of PANDAS children do not need IVIG on a continual basis, and repeated high dose (2gram/1kg) IVIG is not recommended by our Scientific Advisory Board. Occasionally a child is found to be clearly immune deficient (PID or CVID). Consultation with an immunologist is important and IVIG follow up may be different.

How Does IVIG Work?

The exact mechanisms of IVIG are not thoroughly understood.

IVIG has been shown to be helpful with the harmful inflammation caused by autoimmune illnesses, but the exact causative actions are not clear yet. Donor antibodies may “retrain” the abnormal antibodies in the patient or the large amounts administered may simply overwhelm the harmful antibodies – thereby removing them from the PANDAS patient.

NIMH IVIG Study

New IVIG study shows 60% mean reduction in symptoms for PANDAS patients.

A new paper (Oct 2016) submitted to the Journal of the American Academy of Child & Adolescent Psychiatry describes the outcome of the NIMH double-blind placebo controlled study of IVIG for treatment of symptoms in children who met the criteria of PANDAS. Read more about it on the Pandas Physicians Network.

Plasmapheresis

Plasmapheresis (Apheresis) or Plasma Exchange (PEX) is a process during which the harmful auto-antibodies are removed from the blood system. This procedure is done in a hospital setting (you can read more about the procedure here).

There are not many providers that offer Plasmapheresis for PANDAS and PANS, but those that do have cited seeing symptoms improve even while the procedure is still occurring. When a child is presenting with very severe symptoms that would be considered life-threatening, Plasmapheresis may be the preferred method of treatment due to the quick response rate. In some cases, PEX has had to be repeated (as with IVIG) . Again, prophylactic antibiotics should be maintained.

The American Society for Apheresis lists Plasma Exchange as an accepted 1st-line therapy, either alone or with other treatment for a PANDAS exacerbation. The 2019 Guidelines from the Journal of Clinical Apheresis can be found here.

The full ASFA guidelines, as established in the 2013 Journal of Clinical Apheresis, appeared in their ‘Special Issue’ that is published every 3 years. The guidelines can be found at: Guidelines on the Use of Therapeutic Apheresis in Clinical Practice—Evidence-Based Approach from the Writing Committee of the American Society for Apheresis: The Sixth Special Issue.

Merck Manual on Therapeutic Apheresis also lists Plasma Exchange as a first line therapy at http://bit.ly/1JcvSth

Other Treatments

CBT/ERP

CBT (Cognitive behavioral therapy) and/or ERP (Exposure and Response Prevention) may be beneficial for a recovering PANDAS child. Medical interventions, such as antibiotics, IVIG, etc, are needed to lay a foundation for therapy to be introduced.

Some information on CBT as it pertains to PANDAS and PANS, can be found in Dr. Eric Storch’s presentation CBT for PANDAS and PANS(2012).

Steroids

Steroids likely reduce the inflammation occurring in the child’s brain and have been shown to reduce severity of symptoms in patients with Sydenham’s chorea. Active infections need to be addressed with the use of steroids. Some children with PANDAS/PANS can experience a worsening of tics and/or aggression. It is important to discuss with your provider the pros and cons of using the steroid.

Tonsillectomy

A tonsillectomy may be considered by an experienced ENT. Some research has shown marked improvement post tonsillectomy, including full cessation of symptoms in some patients. This has been noted in both a 2008 case study and 2015 JAMA Case Series. If a full remission of symptoms does not take place immediately following the procedure, it does not mean the surgery will not have any benefits. Removing the tonsils should lower the chances of a person contracting strep. A child may also see a more gradual improvement post-surgery.

A treatment course of antibiotics prior to surgery and antibiotics post-surgery can be beneficial. Requesting the tonsils be biopsied post-surgery may also show whether strep, staph, or other bacteria was hiding in the tonsillar crypts.

Omega 3's

Some children are advised to take an Omega 3 supplement because of its known positive effect on brain function. It is also known to reduce inflammation and reduce hyperactivity and increase focus in children with ADHD.

Ibuprofen

Ibuprofen is classified as a NSAID (nonsteroidal anti-inflammatory drug). Some children have a temporary easing of symptoms with Ibuprofen.

If a child does not show any improvement with Ibuprofen, this does NOT dismiss a possible PANDAS or PANS diagnosis. Ibuprofen should only be given under the direction of a physician.

Probiotics

Probiotics help maintain the healthy gut bacteria, which can be compromised with antibiotic use. Probiotics should be taken a minimum 2 hours apart from an antibiotic.